Idiopathic Hyperammonemia after Orthotopic Lung Transplantation - Juniper Publishers - Journal of Anesthesia & Intensive Care Medicine
Juniper Publishers - Open Access Journal of Journal of Anesthesia & Intensive Care Medicine
Idiopathic Hyperammonemia after Orthotopic Lung Transplantation
Authored by Tyson Kathleen
Idiopathic hyperammonemia is characterized by
increased serum ammonia levels (>200umol/L) and is sometimes
associated with normal to slightly elevated liver function tests [1].
These patients often present with encephalopathy, cerebral edema,
seizures, and coma. Idiopathic hyperammonemia has been reported after
high dose chemotherapy, and organ transplants including Orthotopic lung
transplant [2]. Hyperammonemia is a rare, severe, and often fatal
complication. There are few cases reported in the literature since 1997,
including one retrospective analysis of 807 lung transplants; only 8
patients were diagnosed with hyperammonemia [3]. The exact mechanism of
idiopathic hyperammonemiais not fully understood with congenital or
acquired defects in ammonia metabolism, as well as bacterial infection
having been described [3].
Ammonia is primarily cleared by the liver’s urea
cycle. The urea cycle converts ammonia to urea through a series of
intermediate reactions. Hyperammonemia can be the result of any number
of inborn errors (congenital or acquired) of urea cycle metabolism.
Another hepatic mechanism for ammonia clearance is through glutamate
synthetase, which converts glutamate and ammonia to glutamine. In the
kidneys, glutamine is broken down back into glutamate and ammonia, which
is then excreted. If hepatic or renal elimination of ammonia is
interrupted, then hyperammonemia can occur leading to severe
consequences. We present a case below of a female patient who developed
hyperammonemia after Orthotopic lung transplant.
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